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Related to cleft lip: spina bifida
Cleft palate and cleft lip result from failure of the two sides of the face to unite properly at an early stage of prenatal development. The defect may be limited to the outer flesh of the upper lip (the term harelip, suggesting the lip of a rabbit, is both inaccurate and unkind), or it may extend back through the midline of the upper jaw through the roof of the palate. Sometimes only the soft palate, located at the rear of the mouth, is involved.
The infant with a cleft palate is unable to suckle properly, because the opening between mouth and nose through the palate prevents suction. Feeding must be done by other means, with a dropper, a cup, a spoon, or an obturator, a device inserted in the mouth to close the cleft while the baby is sucking. Cleft palate allows food to get into the nose, and it causes difficulty in chewing and swallowing. Later it will hinder speech, because consonants such as g, b, d, and f, which are normally formed by pressure against the roof of the mouth, are distorted by resonance in the nasal cavity. The cleft may also prevent movements of the soft palate essential in clear speech.
cleft lipEmbryology A congenital defect characterized by a failure in the fusion of the upper lip; often associated with cleft palate. See Cleft palate.
cleft lip(kleft lip)
Cleft Lip; Cleft Palate
|Mean LOS:||2.2 days|
|Description:||SURGICAL: Other Ear, Nose, Mouth, and Throat Operating Room Procedures Without CC or Major CC|
Cleft lip (CL) and cleft palate (CP) are facial malformations of the upper lip or palate that are the fourth most common congenital abnormality in the United States. They may appear separately or, more commonly, together. The malformation is a result of a failure of the maxillary and median nasal processes to fuse during the second month of embryonic development. CL may vary from a small notch to a complete cleft that extends into the base of the nose. When CP occurs alone, it is midline, but when it occurs with CL, it may extend into either side of the soft palate. Related complications of CL/CP include dental malformations, frequent otitis media leading to hearing impairment, speech difficulties, and social isolation due to poor self-image and speech impairments.
A genetic cause for CL/CP is likely; however, environmental exposure to teratogens during critical embryonic development cannot be ruled out. CL with or without CP is etiologically and genetically distinct from isolated CP. Isolated CP has a greater incidence of associated anomalies. CP may also be one of the three signs of Pierre Robin sequence, which also is associated with micrognathia and glossoptosis. There is a two-fold increase in the occurrence of clefts with maternal smoking in early pregnancy.
There are over 400 single-gene, complex disorders, or syndromes, that include CL and/or CP as features. Approximately 22% of facial clefting can be attributed to single-gene disorders. The gene IRF6 has been identified as a probable cause in approximately 12% of CLs and may account for familial trends. Familial CP appears to have an autosomal dominant pattern of transmission. Overall CL/CP heritability is estimated at approximately 76%. Along with the increased risk for CL/CP in some families, those families may have a greater risk genetically for specific types of cancer (e.g., colon) than families who do not have the associated congenital anomalies of CL/CP.
Gender, ethnic/racial, and life span considerations
The incidence of CL with or without CP varies by race, with a higher rate among Japanese and certain groups of Native Americans and a lower rate among African Americans. Infant males are more likely to have CL with or without CP, whereas females are more likely to have CP alone.
Global health considerations
A variety of genetic, environmental, and nutritional causes affect global prevalence. People in developing countries with poor nutrition or environmental exposure to toxins may have a higher risk for CL and CP than people in developed countries.
A family history of CL or CP may or may not exist. Identical twins are more likely to share the disorder than are fraternal twins. Inquire about teratogen exposure during the first trimester of pregnancy.
The primary symptoms are facial malformations of the upper lip or palate. The CL may vary from a small notch to a widespread open cleft and may be unilateral or bilateral. The CP also varies in the extent of the malformation: It can involve only the uvula, extend into the soft and hard palate, or be unilateral, bilateral, or midline.
Parents’ and families’ adjustments to an infant with CL or CP may be difficult. The deformity is usually readily observable at birth and often totally unexpected. Support for the family is essential and includes explanations of the surgical procedures and long-term prognosis.
There are no diagnostic tests for CL or CP. CL is diagnosed by visual inspection. CP is diagnosed by palpating the palate with a gloved finger during the initial newborn assessment at birth. Inspect the palate during crying. It is possible today to diagnose the presence of CL and CP in utero with an ultrasound. Three-dimensional ultrasound can be used to assess fetal faces accurately. Facial x-rays may be used to confirm the extent of bone deformity.
Primary nursing diagnosis
DiagnosisAltered nutrition: Less than body requirements related to inadequate intake
OutcomesNutritional status; Food and fluid intake
InterventionsBottle feeding; Infant care
Planning and implementation
surgical.CL and CP are treated with a combination of surgery, speech therapy, and orthodontic work. Surgical repair of a CL is performed within the first month after birth. The repair improves the child’s ability to suck. The optimal time to surgically correct a CP is controversial. Times range from 28 days of life to 18 months. Most surgeons prefer to perform the surgery at an early age before faulty speech habits develop. The more extensive the surgery required, the later the surgery may occur. Surgical repair of CL (cheiloplasty) is usually uncomplicated with no long-term intervention other than possible scar revision. Surgical repair of CP (palatoplasty) is more extensive and may require more than one surgery. Anesthesia for repair of CL/CP needs thoughtful consideration due to the proximity of surgery to the airway. If the infant has horseshoe defect, surgery may be impossible. A contoured speech bulb attached to the back of a denture appliance to occlude the nasopharynx may help the child speak.
|Medication or Drug Class||Dosage||Rationale|
|Antibiotics||Depends on the drug and weight of the child||Prevent infection resulting from surgery|
|Analgesics||Depends on the drug and weight of the child||Relieve surgical pain|
Experimental Treatment: Surgical repair of CL in animals in utero demonstrated better healing of the lip than conventional means.
Because of the long-term, multidisciplinary nature of services needed for the child, assist the parents in accessing appropriate support within the healthcare system. Support the parents before and during the surgical procedure by identifying the positive features of the newborn. Call the infant by name. Current surgical practices provide excellent repairs with minimal scarring. Encourage parents to discuss their feelings about the child’s appearance. Sharing pictures of children with successful CL repairs may help the parents cope with their fears and anxieties.
Depending on the severity of the cleft, children with CL/CP will have problems sucking. Work with the parents and experiment with devices that will improve nutrition, such as different kinds of nipples. The infant may feed better if the parents use a nipple with a flange that occludes the cleft or a large, soft nipple with large holes. Try holding the infant at different positions during feeding (e.g., at a 60- to 80-degree angle). Breastfeeding can be successfully carried out as long as the mother can maintain a seal during nursing. In some nursing pairs, the breast tissue may help form the seal. Otherwise, the mother can use the hand not holding the infant or she can use a molded nipple. Encourage discharge follow-up to support the breastfeeding mother-baby dyad.
Parents should be allowed to verbalize fears and anxiety about the deformity. The first time parents see their baby, they may experience shock, disappointment, or guilt. If you help them see the baby’s assets, you encourage bonding and acceptance. Allow ample time for the parents to hold the infant to promote bonding. Explain the surgical procedure and postoperative care to parents.
The postoperative management of an infant with a CL focuses on protection of the operative site. Surgical closure with Dermabond has demonstrated fewer infections and hypertonic scar repair than Steri-strips. Arm restraints prevent the child from rubbing the site and from self-injury. Hang baby toys within reach of the baby’s restrained hands. Many infants are more comfortable in an infant seat rather than lying in a crib. To avoid facial contact with the sheets, do not place infants on their stomachs for sleep. Pacifiers are contraindicated, and feeding methods should be designed to reduce any tension on the suture line. Use a cotton-tipped applicator and a cleansing solution to clean the suture line. An antibiotic ointment may be prescribed. Pain should be controlled with analgesic medication and nonpharmacologic strategies, such as holding and rocking.
The postoperative management of an infant with a CP centers on prevention of injury and infection to the operative site. Do not place sharp or potentially injurious objects in the child’s mouth (spoons, forks, straws, etc.). Feeding may be done from the side, but self-feeding is prohibited. After feeding, make sure to cleanse the child’s mouth with water or a cleansing solution.
Evidence-Based Practice and Health Policy
Reilly, S., Reid, J., Skeat, J., Cahir, P., Mei, C., & Bunik, M. (2013). ABM Clinical Protocol #17: Guidelines for breastfeeding infants with cleft lip, cleft palate, or cleft lip and palate, revised 2013. Breastfeeding Medicine, 8(4), 349–353.
- Breastfeeding may not be initiated immediately in infants with cleft lip or palate because they have difficulty creating the suction necessary for feeding.
- However, evidence-based recommendations include promoting breast milk by other methods, such as a bottle or spoon if the infant is unable to establish adequate sucking directly from the breast. Modifications to breastfeeding positions and possible delayed transitioning to the breast should also be discussed.
- Additional recommendations propose that infants with CL or CP should be evaluated on an individual basis for breastfeeding readiness, and mothers who wish to breastfeed should be given immediate access to a lactation specialist.
- Monitoring the infant’s hydration status and growth is recommended while an adequate feeding pattern is being established, in addition to introducing breastfeeding immediately following CL repair and 1 day after repair of a CP.
- Appearance of surgical site: Presence of redness, drainage, swelling; degree of approximation of wound edges
- Response to pain medication and other nonpharmacologic interventions
- Ability to feed and maintain weight
Discharge and home healthcare guidelines
feeding.Teach the parents feeding techniques, how to observe for aspiration, and to bubble the infant frequently. After surgery, teach the parents to avoid putting objects into the infant’s mouth.
prevention of infection.Teach the parents to care for the incision and to assess the incision for infection. Explain the importance of keeping the infant’s hands away from the face. Tell the parents that it is important to hold the infant and remove the restraints from time to time.
pain control.Teach the parents the signs of pain in an infant and explore with them nonpharmacologic methods to relieve pain. Review with the parents the analgesic medication dosage, time, and route.
complications.Instruct parents that the child may have more recurrent middle ear infections than other children. The child may also need orthodontic or speech therapy at some time because of the deformity of the mouth and palate.
cleft lip(kleft lip)